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Peripheral primitive neuroectodermal tumor of the pelvis.

Zohreh Yousefi¹, Nourieh Sharifhi², Malihe Hasanzadeh³

¹Cancer Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran;

Iranian Journal of Medical Sciences

|January 24, 2014

Summary

Primitive neuroectodermal tumors (PNETs) are rare, highly malignant small round cell tumors. This study presents three pelvic PNET cases, highlighting their aggressive nature and the need for inclusion in pelvic mass differential diagnoses.

Keywords:

Broad ligament Ewing’s sarcoma Ovary Pelvic neoplasm Primitive neuroectodermal tumors

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Area of Science:

Oncology
Pathology
Surgical Oncology

Background:

Primitive neuroectodermal tumors (PNETs) are aggressive, malignant small round cell neoplasms.
PNETs typically arise in bone and soft tissues but can occur in the pelvis.
PNETs share characteristics with Ewing sarcoma and are of neuroectodermal origin.

Observation:

Three rare cases of pelvic primitive neuroectodermal tumors are presented.
These tumors occurred in the ovary and broad ligament.
PNETs exhibit aggressive clinical behavior and poorer outcomes compared to other small round cell tumors.

Findings:

Tumor site, volume, and metastasis presence are significant prognostic factors for PNET.
Multimodal treatment, including surgery and chemotherapy, is the standard protocol.
Pelvic PNETs represent a rare but important diagnostic consideration for pelvic masses.

Implications:

Increased awareness of pelvic PNETs is crucial for accurate diagnosis and timely treatment.
Early detection and appropriate management can potentially improve patient outcomes.
Further research into the specific characteristics and optimal treatment of pelvic PNETs is warranted.